Subacute cutaneous lupus erythematosus with pityriasis-like cutaneous manifestations

A 57-year-old woman was seen in our department in September 1999. At the time of our observation, she complained of fever and malaise and presented many erythematous and squamous lesions on sun-exposed areas. On the upper third of the back (Figs 1, 2), she presented a wide patch with irregular borders, due to many coalescing small purplish-red lesions. Each lesion was covered with branny scales and, on palpation, revealed a variable degree of infiltration. Few areas of uninvolved skin could be seen inside the main patch. Beyond this main patch, small individual scattered lesions with a net-like distribution were detected. Similar lesions were present on the neck, chest, and arms. Her face showed diffuse erythema with fine scaling, except for the nasolabial folds which were unaffected. Telangiectasias were present on her face, trunk, limbs, and periungual areas. The patient stated that such lesions had developed after sun exposure. In 1990, a diagnosis of systemic lupus erythematosus (SLE) was made according to the presence of five American Rheumatism Association (ARA) criteria: photosensitivity, malar eruption, diffuse arthralgias (associated with swelling of the finger joints), mild renal involvement with proteinuria, and leukopenia. Since then she has also presented anti-Ro/ SSA antibodies, hypertension, and Raynaud's phenomenon. A histologic study performed on the skin of the back showed a moderate epidermal atrophy with a slight hyperorthokeratosis, a pronounced hydropic degeneration of the basal cell layer, an intense band-like and perivascular lymphocytic infiltrate, and a moderate diffuse edema of the upper dermis (Fig. 3). Laboratory analysis revealed positive anti-dsDNA (titer 1 :160) and anti-Ro/SSA antibodies, iron-deficiency anemia, leukopenia (3000/mm(3)), microhematuria, and persistent proteinuria (0.7 g/day). Hypocomplementemia (C3, 47 mg/dL; C4, 10.4 mg/dL) and increased immunoglobulin G (IgG) serum levels were found, while circulating immune complexes could not be demonstrated. The creatinine clearance was found to be decreased (53 mL/min). Chest X-ray and echocardiography were negative, but a holter examination revealed an abnormal ventricular rhythm. Direct immunofluorescence performed on lesional skin of the back revealed a granular deposition of IgM, C3 at the dermal-epidermal junction (DEJ), a perivascular deposition of C3, and dust-like particles of IgG in the epidermis. We confirmed the previous diagnosis of SLE and classified the unusual clinical manifestations as pityriasis-like subacute cutaneous lupus erythematosus (SCLE). The patient was treated with systemic and local steroids (prednisone, 0.5 mg/kg/day) with a marked improvement of the cutaneous manifestations.